USP19

Protein ubiquitination controls many intracellular processes, including cell cycle progression, transcriptional activation, and signal transduction. This dynamic process, involving ubiquitin conjugating enzymes and deubiquitinating enzymes, adds and removes ubiquitin. Deubiquitinating enzymes are cysteine proteases that specifically cleave ubiquitin from ubiquitin-conjugated protein substrates. This protein is a ubiquitin protein ligase and plays a role in muscle wasting. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.

Full Name

Ubiquitin Specific Peptidase 19

Function

Deubiquitinating enzyme that regulates the degradation of various proteins. Deubiquitinates and prevents proteasomal degradation of RNF123 which in turn stimulates CDKN1B ubiquitin-dependent degradation thereby playing a role in cell proliferation. Involved in decreased protein synthesis in atrophying skeletal muscle. Modulates transcription of major myofibrillar proteins. Also involved in turnover of endoplasmic-reticulum-associated degradation (ERAD) substrates. Regulates the stability of BIRC2/c-IAP1 and BIRC3/c-IAP2 by preventing their ubiquitination. Required for cells to mount an appropriate response to hypoxia and rescues HIF1A from degradation in a non-catalytic manner. Plays an important role in 17 beta-estradiol (E2)-inhibited myogenesis. Decreases the levels of ubiquitinated proteins during skeletal muscle formation and acts to repress myogenesis. Exhibits a preference towards 'Lys-63'-linked ubiquitin chains.

Biological Process

Biological Process negative regulation of skeletal muscle tissue development Source:UniProtKB
Biological Process positive regulation of cell cycle process Source:UniProtKB
Biological Process protein deubiquitination Source:ParkinsonsUK-UCL1 Publication
Biological Process protein stabilization Source:ParkinsonsUK-UCL1 Publication
Biological Process regulation of cellular response to hypoxia Source:UniProtKB1 Publication
Biological Process regulation of ERAD pathway Source:ParkinsonsUK-UCL1 Publication
Biological Process regulation of protein stability Source:UniProtKB
Biological Process response to endoplasmic reticulum stress Source:UniProtKB1 Publication
Biological Process ubiquitin-dependent ERAD pathway Source:UniProtKB1 Publication

Cellular Location

Endoplasmic reticulum membrane

Topology

Cytoplasmic: 1-1291
Helical: 1292-1312
Lumenal: 1313-1318